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Biomedical subjects

E Esscher

Publications and source records attributed to E Esscher.

14 recordsLinked to original sources
PubMed1996

Non-progressive ataxia: origins, brain pathology and impairments in 78 swedish children.

This population-based study refers to 78 Swedish children with non-progressive ataxia from a total population of 3.1 million inhabitants. Inclusion criteria were ataxic gait without any signs of spasticity, dyssynergia, dysmetria and intention tremor. CT and/or MRI studies were available from 70 patients (90%). Infratentorial pathology was revealed in 27%, and findings were considered normal in 61%. If CT was normal, of recent date and of good quality, MRI did not add any new information. In half of the cases with pathological CT, however, MRI provided new information. The origin was considered prenatal in 45% (familial in 17%), perinatal in 4% and unclassifiable in 51%. 60% were mentally retarded; in the rest, cognitive development was near normal (18%) or normal (22%). Speech development was delayed in 88%, and 58% had visual dysfunction.

Adolescent↗
PubMed1986

Maternal antibodies against fetal cardiac antigens in congenital complete heart block.

An immunologic basis for congenital heart block has been proposed previously. To investigate the association between congenital heart block and maternal antibodies capable of crossing the placenta, we used immunofluorescence to examine serum samples from 41 mothers and 8 affected children, together with serum from controls, for antibodies to fetal cardiac tissue. Twenty-one mothers (51 percent) had IgG antibody reactive with fetal heart tissue, as compared with only 9 of 94 controls (10 percent; P less than 0.001). Three of 8 affected babies, but none of 50 healthy babies, had similar antibodies. The antibodies reacted with all myocardial tissue and were not directed specifically to the conduction system. They also reacted with other fetal tissues and could be distinguished from nuclear and smooth-muscle autoantibodies. We also observed a higher occurrence of antibodies to cytomegalovirus, but not to Epstein-Barr virus, in these mothers. Autopsy specimens from babies with congenital heart block examined by immunoperoxidase staining showed deposition of immunoglobulin and complement components in all cardiac tissues. These findings strengthen the case implicating immune reactivity related to maternal antibody in the development of some but not all cases of congenital heart block.

Antibodies, Antinuclear↗
PubMed1986

Anatomy of congenital complete heart block and relation to maternal anti-Ro antibodies.

The anatomy of the conduction system was studied in 8 patients who had isolated congenital complete heart block. Atrial-axis discontinuity was seen in 7 hearts from children born to mothers with anti-Ro antibodies. The anticipated area of the atrioventricular node was occupied by fibrous and adipose tissue. The heart from the only child whose maternal serum was anti-Ro negative had nodoventricular discontinuity of the conduction system. This difference in anatomy may have etiologic significance.

Antibodies↗
PubMed1983

Connective-tissue disease, antibodies to ribonucleoprotein, and congenital heart block.

The relation between congenital heart block and maternal connective-tissue disease was studied by antibody screening of serum samples obtained in connection with 45 cases of isolated congenital complete heart block. Serum was available from 41 mothers (17 who had connective-tissue disease and 24 who were healthy) and 21 children. Thirty-four mothers had antibody to a soluble tissue ribonucleoprotein antigen called Ro(SS-A), which was identified by immunodiffusion. Anti-Ro(SS-A) was found in seven of eight serum samples collected from affected children when they were less than three months old but in none of 13 samples obtained when these children were older. It appears that maternal anti-Ro(SS-A) antibody crosses the placenta and is a marker for risk of congenital complete heart block; its absence from maternal serum suggests that a child is unlikely to be affected. Anti-Ro(SS-A) or a related antibody is probably involved in the pathogenesis of congenital complete heart block.

Autoantibodies↗
PubMed1981

Review article. Congenital complete heart block.

Isolated congenital complete heart block has a heterogenous aetiology. In approximately one-third of the patients in the present material the mothers had symptoms or signs of connective tissue disease. The mortality is highest in the neonatal period, much lower during childhood and adolescence and increases slowly later in life. There are elderly patients who are completely asymptomatic but patients are also encountered with signs and symptoms of more or less severe myocardial damage. Stokes-Adams attacks may occur at any age. About 10% of patients below 15 years of age in this material and about 25% of those above 15 years (mean age 30 years) were paced. In the neonatal period the predominant indication for pacing was heart failure. It is difficult to make a prognosis in the individual patient. Apart from already known risk factors, a fixed or decreasing low ventricular rate neonatally and a prolonged QT time seem to be bad prognostic signs.

Age Factors↗
PubMed1979

Congenital heart block and maternal systemic lupus erythematosus.

The association between infants with congenital heart block (CHB) and the presence or later development of maternal systemic lupus erythematosus or other connective-tissue disease (CTD) was reviewed in 67 cases. In 24 cases CHB was diagnosed at or before birth. Of nine necropsies on affected infants, seven showed endomyocardial fibrosis. The results suggest that one in three mothers who deliver babies with CHB have or will develop CTD. The association is probably explained by placental transfer of a maternal antibody. Awareness of the association may lead to prevention of the birth of children with CHB and better neonatal care of affected children.

Adolescent↗
PubMed1977

Long-term results following closure of isolated ostium secundum atrial septal defect in children and adults.

Information is presented on 166 patients operated for isolated atrial septal defect of secundum type with a mean follow-up time of 12 yr (range 8-21 yr). The mean age at operation was 23 yr (range 4-63 yr). The incidence of suspected mitral regurgitation is very low (4 cases). Postoperative flutter or fibrillation occurred in 24 cases, 20 out of these were operated after the age of 20 yr. In all 24 cases intermittent or permanent atrial dysrhythmia was present immediately postoperatively. The long-term results with regard to the occurrence of dysrhythmias and increased heart volumes is better in the younger age group.

Adolescent↗
PubMed1975

Familial, isolated, complete right bundle-branch block.

An electrocardiographic study of the families of two children with isolated complete right bundle-branch block is presented. Several cases of classical complete right bundle-branch block were disclosed (17/69 and 3/22 respectively) in three generations of the two families. Personal interviews and parish registers showed that the two families were related, being descendants 8 generations back from a glass-blower who has emigrated to Sweden in the eighteenth century. It is concluded that complete right bundle-branch block can be an autosomal, genetic anomaly which is inherited dominantly but with reduced penetrance and/or expressivity.

Adolescent↗
PubMed1975

Cardiovascular malformation in infant deaths. 10-year clinical and epidemiological study.

The infant mortality from cardiovascular malformations in a region with a population of 2 million inhabitants during a 10-year period has been studied. The study involved validation of the diagnoses and judgement whether the cardiovascular malformation was the dominating or a contributing cause of death. It is shown that the incidence of fatal cardiovascular malformations is probably overestimated in the official death statistics. Evaluation of the clinical findings and necropsy reports are important aids in obtaining more reliable figures of the incidence. The rate of referral of infants with cardiovascular malformations has increased during the period of this study, so that the number of operable lesions not referred has decreased. The infant mortality rate, found in this study, of 1.33 per 1000 liveborn babies constitutes about 20 per cent of all liveborn infants with cardiovascular malformations. The most common lesions found in those who died belonged to the group constituting the hypoplastic left heart syndrome. The proportion of this type of malformation, about 20 per cent of all those dying, is higher than in other similar studies. This difference can probably be explained by variations in selection and classification.

Aortic Coarctation↗
PubMedDate not supplied

Q-T interval in congenital complete heart block.

The rate-corrected Q-T interval (Q-Tc) was measured in 273 children with congenital complete heart block. Fifty-nine had prolonged Q-Tc and 50 of these had symptoms. All 10 with isolated complete heart block, prolonged Q-Tc and bundle branch block had symptoms. The ventricular rate (VR) was significantly lower in patients with prolonged Q-Tc than in their age-matched controls. The outcome is worse for children with low VR and long Q-Tc than for those with low VR and normal Q-Tc. In 11 children a prolonged Q-Tc preceded symptoms by more than 1 month. This suggests that prolonged Q-Tc in children with congenital complete heart block can be of prognostic significance.

Adams-Stokes Syndrome↗